Arterial Dissection Spontaneous Causes: Fibromuscular Dysplasia and Marfan Syndrome

Early diagnosis of arterial dissection helps in preventing lesions which may be permanent neurologic lesions that could affect the quality of life or may even lead to death among patients. The increased incidence of cerebral ischemic accidents due to this case can be accounted to poor diagnosis.


Neurologic Symptoms of Arterial Dissection

The most frequent symptoms reported by patients who are suffering from arterial dissection are mostly neurological signs such as headache, facial pain or cervical pain with acute onset with headache being the most frequent symptom in 55% to 100% of patients. Headache and facial pain after dissections are usually unilateral, severe and persistent, in average lasting four days and gradually improving in up to 30 days.

Early identification is critical to prevent cerebral ischemic accidents and to improve patients’ prognosis. Aside from the classic symptoms of arterial dissection, it is important to classify the causes of arterial dissection.

Here are just 2 of the classified Spontaneous causes of arterial dissection:

a. Fibromuscular Dysplasia

b. Marfan Syndrome

Fibromuscular Dysplasia (FMD)

Blood vessels labelledFibromuscular Dysplasia is a heterogeneous group of vascular lesions characterised by an idiopathic, non-inflammatory, and non-atherosclerotic angiopathy of small and medium-sized arteries. This is frequently asymptomatic but the symptomatic patients commonly present with arterial dissection due to carotid and vertebral artery involvement. It is the weakening of the vessels caused by FMD which predisposes it to arterial dissection.

Marfan Syndrome (MFS)

Studies and case reports published in the early 80s show that patients with spontaneous arterial dissection may also have a history of stroke and/or hereditary connective tissue disorders like Marfan Syndrome.

Marfan Syndrome (MFS) is a genetic disorder of the connective tissue. They are noticeable to have long disproportionate extremities and appear to be tall. They may also experience upward and lateral optic lens dislocation. Cardiovascular disease is common, particularly aortic root dilatation and dissection which is the most common cause of sudden death in these patients.

The Case of Jonathan Larson:

Jonathan Larson, an American composer and playwright of the famous broadway musical RENT died unexpectedly the morning of Rent’s first preview performance Off Broadway due to aortic dissection. His undiagnosed Marfan Syndrome may have caused the aortic dissection. The New York State medical investigators believed that he would have lived if his aortic dissection had been properly diagnosed and treated.

Prior to his death, Larson was noted to have been suffering severe chest pains, dizziness, and shortness of breath for several days. However, doctors could not find signs of a heart attack even after doing a chest X-ray and electrocardiogram so they misdiagnosed it either as flu or stress.

Currently, Marfan Syndrome has no cure although many people have a normal life expectancy with proper treatment.

As arterial dissection incidence increases and recognized as a cause of stroke that affects many people both via spontaneous or traumatic causes, it is important to understand that proper diagnosis and treatment plays an important part in helping save the lives of your loved ones.

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